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Objective Serum amino-terminal propeptide of C-type natriuretic peptide ( NT-pro CNP) levels have been proposed as a biomarker of linear growth in healthy children. The usefulness of NT-pro CNP in patients with achondroplasia ( ACH)/hypochondroplasia ( HCH) remains to be elucidated. The objective was to study whether serum NT-pro CNP level is a good biomarker for growth in ACH/ HCH and other patients of short stature. Design This was a longitudinal cohort study. Patients Sixteen children with ACH (aged 0·4-4·3 years), six children with HCH (2·7-6·3 years), 23 children with idiopathic short stature ( ISS) (2·2-9·0 years), eight short children with GH deficiency ( GHD) (2·9-6·8 years) and five short children born small for gestational age ( SGA) (2·0-6·6 years). Patients with ACH/ HCH received GH treatment for 1 year. Measurements Serum NT-pro CNP levels and height were measured. Results NT-pro CNP levels positively correlated with height velocity in these short children ( P < 0·05, r = 0·27). NT-pro CNP levels inversely correlated with age in children with ISS alone ( P < 0·01, r = −0·55). Serum NT-pro CNP levels in patients with ACH/ HCH were increased 3 months following the initiation of GH treatment ( P < 0·05). Height SDS gain during GH treatment for 1 year was positively correlated with the changes in NT-pro CNP levels after the initiation of GH ( P < 0·01, r = 0·72). Conclusion Serum NT-pro CNP levels may be a good biomarker to indicate the effect of GH treatment on growth in patients with ACH/ HCH at least in the first year and height velocity in short stature patients. [ABSTRACT FROM AUTHOR]